Tag: interventions

Let’s talk today about the story of NDPH, a primary headache not well understood or recognized, a zebra among headaches. The more we know about it, the more it is recognized. But that does not make it any easier to deal with as a provider or a patient.

The first documented description of NDPH was in 1986, by Dr. Walter Vanast, a neurologist in Canada. He described it as a benign syndrome that resolved regardless of treatment in 73-86% in 3-24 months. In a recent interview with Dr. Vanast in Headache journal, he describes his interest and search for the cause of this headache. He thought it was an auto‐immune disorder with a persistent viral trigger, such as EBV. And this description is still in use today, though in my opinion ‘benign’ is not the word I would use.

About half of patients with NDPH have had a febrile viral illness near the onset. Others develop it after a minor head injury, or pseudotumor or idiopathic intracranial hypertension (IIH). Others seem to have no known reason for this headache to develop. There continues to be very little known about the pathophysiology of NDPH. A recent paper stated that it might be related to persistent central nervous system inflammation or related to cervical hypermobility. We really don’t know.

The incidence in adult of chronic daily headache is 4%; the incidence of these adult patients having NDPH is between 1-10%, so not that many. But in children and teens, the incidence is much higher: 20-40% of pediatric chronic daily headache patients. Way more kids and teens than adults have NDPH. And it is probably underreported and under recognized.

There are several different patterns of headache associated with NDPH.

The first type is mild and self-limiting, resolving without therapy.

The second type is moderate, with a constant headache that waxes and wanes over time. Good days are more like tension-type headaches and bad days are more like migraine.

The third type is a continuous severe headache, with disabling high-level pain all day every day.

We don’t see many patients with the first type, with lower level headaches that just end up going away. Most commonly we see the second type, kids with constant all day every day headache, pain rated 5-6/10NRS on average with a range of 3-9/10NRS. On lower pain days, they might have mild nausea, mild dizziness, mild mental fogginess and light sensitivity along with headache. On higher pain days, all of their symptoms are severe and migrainous; pain is 8-9/10NRS, and they are non-functional. Their pain and symptoms are disabling; change in pain level is influenced by activity, weather patterns, stress and cognitive effort. Fortunately, while we do see the completely refractory third type, they are not as common– but so difficult.

So it is hard enough to have a constant headache, no headache-free time. But add to that is the realization that most common treatments and even the most aggressive treatments are ineffective against this headache. Current daily medications do not alleviate their pain and the patients just experience all the side effects.

Usually by the time the patient arrives at our specialty headache program, they have already tried at least 1 or more daily medications without success. The families might like to try more medication, so we would pick another of the common ones. The choice is often based on what other symptoms are most bothersome.

Unable to sleep? Choose amitriptyline or gabapentin.

Worried about weight gain? Choose topiramate.

Don’t want any mental clouding? Try propranolol.

Super dizzy? Try verapamil.

Persistent nausea? Add hydroxyzine at bedtime.

Very tight neck and upper back muscle? Choose tizanidine.

Don’t want medications? Try a supplement- magnesium or riboflavin.

All else fails? Try occipital nerve and trigger point injections.

You might find that something helps other symptoms, even when it does nothing to the headache. I have had great results with using hydroxyzine at bedtime for patients with persistent nausea. Having less nausea may make the difference between going to school or staying home…. again.

It is just a matter of time before the families (really the patients) decide that they do not want to try any further medication, at least daily medication. Most tell me that they feel better off the meds.

Things you might try for a migraineur in status migrainosis such as going to the ED or being admitted for DHE are not usually helpful. Often the patients end up more frustrated, faced with another ineffective intervention.

As far as rescue medications, again you try the basics- NSAIDS, antiemetics, caffeine. Most important is to stress that analgesia should only be used for severe headache and no more than 3 days/week to avoid overuse headache. As you can imagine, overuse is tempting, kids just want to feel better. But I stress that too much analgesia will just make them feel worse, and in the end, the medications will stop working. I encourage being strategic with analgesia. Save it for when you might really need it, such as before SAT exams or before a big sports game or concert. This is generally effective in preventing overuse.

What is really important is for the families and patients to know that you are all in this together. Accepting where they are and working with them. Offering hope but being realistic.

I will have more to say about some research initiatives and novel approaches to NDPH in the next post.